Department of Neurology, Xijing Hospital, Fourth Military Medical University, 127 Changle West Road, Xi'an, 710032, Shaanxi Province, China.
State Key Laboratory of Cancer Biology, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi Province, China.
Neurocrit Care. 2022 Feb;36(1):192-201. doi: 10.1007/s12028-021-01283-4. Epub 2021 Jul 20.
To date, specialized studies focusing on status epilepticus (SE) in anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis are limited, and the association between the occurrence of SE and clinical outcome is controversial. This study aims to investigate the differences between patients with critical anti-NMDAR encephalitis with SE and patients who experienced epileptic seizures without SE and to evaluate the long-term disease outcomes of patients with anti-NMDAR encephalitis with SE who were admitted to the neurological intensive care unit (neuro-ICU).
In this retrospective study based on a prospective registry, patients with anti-NMDAR encephalitis with neuro-ICU admission from 2014 to 2019 were analyzed and divided into two groups based on whether they had SE. Their clinical characteristics during the neuro-ICU stay were assessed and compared. The neurological and seizure outcomes were evaluated every 3 months.
Of 83 patients with anti-NMDAR encephalitis, 24 required intensive care. In the SE group, 38.5% (5 of 13) of patients developed refractory SE (RSE), and 21.3% (3 of 13) of patients developed super RSE. More patients in the SE group presented with seizures as the initial symptoms (53.8% vs. 9.1%, p = 0.033) and had a strong positive NMDAR antibody titer in the cerebrospinal fluid (76.9% vs. 27.3%, p = 0.043). More patients in the non-SE group had a good neurological outcome (modified Rankin Scale (mRS) score < 2) at 3 months after disease onset (60.0% vs. 15.4%, p = 0.039), but 83.3% of patients with SE had a mRS score < 2 at 9 months after disease onset, which was similar to the rate in the non-SE group. A total of 41.7% of patients with SE had their last seizure within 1 month from disease onset, which was significantly lower than the rate in the non-SE group (90%), but all the patients with SE became seizure free after the acute phase of disease.
Patients with critical anti-NMDAR encephalitis who present with SE have a high rate of RSE/super RSE and recover more slowly than patients without SE, but most of them will eventually achieve good long-term neurological outcomes and live seizure free after the acute phase.
目前，专门针对抗 N-甲基-D-天冬氨酸受体（抗 NMDAR）脑炎的癫痫持续状态（SE）的研究有限，并且 SE 的发生与临床结局之间的关联存在争议。本研究旨在探讨伴有 SE 的抗 NMDAR 脑炎患者与无 SE 癫痫发作患者之间的差异，并评估伴有 SE 的抗 NMDAR 脑炎患者入住神经重症监护病房（neuro-ICU）的长期疾病结局。
本研究基于前瞻性登记，对 2014 年至 2019 年入住 neuro-ICU 的抗 NMDAR 脑炎患者进行回顾性分析，并根据是否发生 SE 分为两组。评估和比较两组患者在 neuro-ICU 期间的临床特征。每 3 个月评估一次神经和癫痫发作结局。
83 例抗 NMDAR 脑炎患者中有 24 例需要重症监护。在 SE 组中，38.5%（5/13）的患者发生难治性 SE（RSE），21.3%（3/13）的患者发生超难治性 SE（super RSE）。更多 SE 组患者以癫痫发作为首发症状（53.8% vs. 9.1%，p=0.033），且脑脊液中 NMDAR 抗体滴度呈强阳性（76.9% vs. 27.3%，p=0.043）。非 SE 组患者在发病后 3 个月时具有更好的神经功能结局（改良 Rankin 量表（mRS）评分<2）（60.0% vs. 15.4%，p=0.039），但 83.3%的 SE 患者在发病后 9 个月时 mRS 评分<2，与非 SE 组相似。SE 组中 41.7%的患者在发病后 1 个月内最后一次发作，明显低于非 SE 组（90%），但所有 SE 患者在疾病急性期后均无癫痫发作。
伴有 SE 的抗 NMDAR 脑炎重症患者 RSE/super RSE 发生率较高，恢复较不伴有 SE 的患者慢，但大多数患者最终将获得良好的长期神经结局，并在急性期后无癫痫发作。
